Synonyms

• Cystic hygroma
• Lymphatic malformation
• Cavernous lymphangioma

Category

Soft tissue

Behaviour

Benign

Gender

M = F

Tissue of Origin

Vascular

Epidemiology

• Benign developmental anomaly of lymphatic vessels
• Often presents in childhood, can be congenital
• No Malignant potential
• Can spontaneously regress

Clinical Features

• Painless swelling in head and neck (75%) or axilla
• Soft, compressible mass
• May transilluminate
• Can become infected or inflamed
• Risk of respiratory compromise if cervical

Location

• Neck (posterior triangle most common)
• Axilla
• Mediastinum
• Retroperitoneum
• Pelvis

Imaging

• Ultrasound shows multiple anechoic cysts
• MRI shows fluid-filled cavities with no enhancement
• No mass effect despite size
• T2 hyperintense, T1 hypointense

Pathology

• Thin-walled lymphatic channels
• Endothelial lining
• Fluid-filled cystic spaces
• No smooth muscle in walls

Genetics

• Sporadic in most cases
• Associated with Noonan, Turner, Down Syndromes in some
• No specific genetic alteration

Treatment

• Observation if asymptomatic
• Surgical excision if symptomatic or cosmetically concerning
• Sclerotherapy for cystic lesions
• Medical management of complications

Prognosis

• Excellent prognosis
• No Malignant transformation
• Risk of recurrence after incomplete excision
• Some spontaneous regression possible

Key Points

• Benign lymphatic malformation
• Often congenital but can present later
• Observation reasonable if asymptomatic
• Excellent prognosis with appropriate management

Workup - Local Imaging

• Ultrasound for initial assessment
• MRI for extent and relationship to surrounding structures

Follow-up Summary

• Imaging surveillance if managed conservatively
• Clinical assessment for complications
• No long-term oncologic Follow-up needed